高级别卵巢癌（HGSOCs）通常起源于输卵管的乳突末端，并携带TP53基因突变。相反，低级别卵巢癌（LGSOCs）似乎具有不同的病理学、流行病学和临床特征，应被视为不同的浆膜上皮卵巢癌亚型。我们目前对LGSOC的了解有限，并且治疗通常来源于更常见的HGSOCs，因为缺乏分离的试验数据。LGSOCs的特点是肿瘤生长缓慢，被认为是浆膜边缘卵巢肿瘤的前体。这些癌症常常为雌激素受体阳性，并显示活化的丝裂原激活蛋白激酶通路，连同KRAS和BRAF突变，极少碰到TP53突变。这些特征现在常用于指导治疗决策，并且，因此，治疗的相当一部分是通过内分泌治疗维持，从而平衡疾病稳定和轻微毒性。此外，正在进行新试验，以检验MEK抑制剂与内分泌治疗的联合作用。本文的目的是总结目前的知识，介绍LGSOCs的正在进行的试验努力。版权所有©2022作者。由Elsevier Ltd。保留所有权利。

High-grade serous ovarian cancers (HGSOCs) most commonly arise from the fimbrial end of the fallopian tube and harbor TP53 gene mutations. In contrast, low-grade serous ovarian cancers (LGSOCs) appear to have different pathological, epidemiological, and clinical features and should be seen as a distinct serous epithelial ovarian cancer subtype. Our current understanding of LGSOC is limited, and treatment has generally been derived from the more common HGSOCs due to a lack of separate trial data. LGSOCs are characterized by slow tumor growth and are assumed to develop from serous borderline ovarian tumors as precursors. These cancers are often estrogen-receptor positive and show an activated mitogen-activated protein kinase pathway together with KRAS and BRAF mutations and, rarely, TP53 mutations. These characteristics are now commonly used to guide therapeutical decision making and, consequently, a substantial part of treatment consists of maintenance with endocrine treatment, thus balancing disease stabilization and mild toxicity. Additionally, new trials are ongoing that examine the role of targeted therapies such as MEK inhibitors in combination with endocrine treatments. The purpose of this work is to summarize current knowledge and present ongoing trial efforts for LGSOCs.Copyright © 2022 The Author(s). Published by Elsevier Ltd.. All rights reserved.